Iridocorneal endothelial syndrome | |
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Specialty | Ophthalmology |
Iridocorneal endothelial (ICE) syndromes are a spectrum of diseases characterized by slowly progressive abnormalities of the corneal endothelium and features including corneal edema, iris distortion, and secondary angle-closure glaucoma. [1] [2] [3] ICE syndromes are predominantly unilateral and nonhereditary. [1] [2] [3] The condition occurs in predominantly middle-aged women. [1] [4] [3] Iridocorneal Endothelial (ICE) syndrome presents a unique set of challenges for both patients and ophthalmologists, and effective treatment of this group of rare ocular diseases requires a combination of diagnostic and therapeutic complexity. It's important to understand.
Many cases are asymptomatic, however patients many have decreased vision, glare, monocular diplopia or polyopia, and noticeable iris changes. [2] [5] On exam patients have normal to decreased visual acuity, and a "beaten metal appearance" of the corneal endothelium, corneal edema, increased intraocular pressure, peripheral anterior synechiae, and iris changes. [1] [2] [5]
The exact mechanism is unknown, however there appears to be a component of abnormal corneal endothelium that proliferates onto the iris forming a membrane that then obstructs the trabecular meshwork, leading to iris distortion. [1] [2] Nodule formation can also occur when the abnormal corneal endothelium causes contractions around the iris stroma. [1] Herpesvirus DNA has been identified in some patients following keratoplasty, suggesting the possibility that herpes simplex virus may induce the abnormal endothelialization in the anterior chamber angle and on the surface of the iris. [2] [4] [6]
The Chandler variant of ICE is characterized by pathology on the inner surface of the cornea leading to abnormal endothelial pump function. [2] [5] Other features include possible mild iris changes, corneal edema, and normal to slight elevations in intraocular pressure. [1] [5]
Cogan-Reese variant is characterized by multiple pigmented iris nodules. [2] [5] This variant is most commonly unilateral and seen in middle-aged females. [2]
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Penetrating keratoplasty and endothelial keratoplasty can be used as treatments for severe cases of ICE. [2] Because glaucoma and elevated intraocular pressure are often present in ICE patients, long term follow up may be needed to ensure adequate intraocular pressures are maintained. [2] [7]
The disease is chronic and often progresses slowly. Prognosis is generally poor when associated with glaucoma. [1] [2]
Eye surgery, also known as ophthalmic surgery or ocular surgery, is surgery performed on the eye or its adnexa. Eye surgery is part of ophthalmology and is performed by an ophthalmologist or eye surgeon. The eye is a fragile organ, and requires due care before, during, and after a surgical procedure to minimize or prevent further damage. An eye surgeon is responsible for selecting the appropriate surgical procedure for the patient, and for taking the necessary safety precautions. Mentions of eye surgery can be found in several ancient texts dating back as early as 1800 BC, with cataract treatment starting in the fifth century BC. It continues to be a widely practiced class of surgery, with various techniques having been developed for treating eye problems.
The corneal endothelium is a single layer of endothelial cells on the inner surface of the cornea. It faces the chamber formed between the cornea and the iris.
A red eye is an eye that appears red due to illness or injury. It is usually injection and prominence of the superficial blood vessels of the conjunctiva, which may be caused by disorders of these or adjacent structures. Conjunctivitis and subconjunctival hemorrhage are two of the less serious but more common causes.
Corneal transplantation, also known as corneal grafting, is a surgical procedure where a damaged or diseased cornea is replaced by donated corneal tissue. When the entire cornea is replaced it is known as penetrating keratoplasty and when only part of the cornea is replaced it is known as lamellar keratoplasty. Keratoplasty simply means surgery to the cornea. The graft is taken from a recently deceased individual with no known diseases or other factors that may affect the chance of survival of the donated tissue or the health of the recipient.
Fuchs dystrophy, also referred to as Fuchs endothelial corneal dystrophy (FECD) and Fuchs endothelial dystrophy (FED), is a slowly progressing corneal dystrophy that usually affects both eyes and is slightly more common in women than in men. Although early signs of Fuchs dystrophy are sometimes seen in people in their 30s and 40s, the disease rarely affects vision until people reach their 50s and 60s.
In ophthalmology, gonioscopy is a routine procedure that measures the angle between the iris and the cornea, using a goniolens together with a slit lamp or operating microscope. Its use is important in diagnosing and monitoring various eye conditions associated with glaucoma.
Corneal dystrophy is a group of rare hereditary disorders characterised by bilateral abnormal deposition of substances in the transparent front part of the eye called the cornea.
Axenfeld–Rieger syndrome is a rare autosomal dominant disorder, which affects the development of the teeth, eyes, and abdominal region.
Polycoria is a pathological condition of the eye characterized by more than one pupillary opening in the iris. It may be congenital or result from a disease affecting the iris. It results in decreased function of the iris and pupil, affecting the physical eye and visualization.
Buphthalmos is enlargement of the eyeball and is most commonly seen in infants and young children. It is sometimes referred to as buphthalmia. It usually appears in the newborn period or the first 3 months of life. and in most cases indicates the presence of congenital (infantile) glaucoma, which is a disorder in which elevated pressures within the eye lead to structural eye damage and vision loss.
Corneal neovascularization (CNV) is the in-growth of new blood vessels from the pericorneal plexus into avascular corneal tissue as a result of oxygen deprivation. Maintaining avascularity of the corneal stroma is an important aspect of healthy corneal physiology as it is required for corneal transparency and optimal vision. A decrease in corneal transparency causes visual acuity deterioration. Corneal tissue is avascular in nature and the presence of vascularization, which can be deep or superficial, is always pathologically related.
Meesmann corneal dystrophy (MECD) is a rare hereditary autosomal dominant disease that is characterized as a type of corneal dystrophy and a keratin disease. MECD is characterized by the formation of microcysts in the outermost layer of the cornea, known as the anterior corneal epithelium. The anterior corneal epithelium also becomes fragile. This usually affects both eyes rather than a single eye and worsens over time. There are two phenotypes, Meesmann corneal dystrophy 1 (MECD1) and Meesmann corneal dystrophy 2 (MECD2), which affect the genes KRT3 and KRT12, respectively. A heterozygous mutation in either of these genes will lead to a single phenotype. Many with Meesmann corneal dystrophy are asymptomatic or experience mild symptoms.
Macular corneal dystrophy, also known as Fehr corneal dystrophy, is a rare pathological condition affecting the stroma of cornea first described by Arthur Groenouw in 1890. Signs are usually noticed in the first decade of life and progress afterwards, with opacities developing in the cornea and attacks of pain. This gradual opacification leads to visual impairment often requiring keratoplasty in the later decades of life.
Fuchs heterochromic iridocyclitis (FHI) is a chronic unilateral uveitis appearing with the triad of heterochromia, predisposition to cataract and glaucoma, and keratitic precipitates on the posterior corneal surface. Patients are often asymptomatic and the disease is often discovered through investigation of the cause of the heterochromia or cataract. Neovascularisation is possible and any eye surgery, such as cataract surgery, can cause bleeding from the fragile vessels in the atrophic iris causing accumulation of blood in the anterior chamber of the eye, also known as hyphema.
Herpetic simplex keratitis is a form of keratitis caused by recurrent herpes simplex virus (HSV) infection in the cornea.
Primary juvenile glaucoma is a subtype of primary congenital glaucoma that develops due to ocular hypertension and is diagnosed between three years of age and early adulthood. It is caused due to abnormalities in the anterior chamber angle development that obstruct aqueous outflow in the absence of systemic anomalies or other ocular malformation.
Pre Descemet's endothelial keratoplasty (PDEK) is a kind of endothelial keratoplasty, where the pre descemet's layer (PDL) along with descemet's membrane (DM) and endothelium is transplanted. Conventionally in a corneal transplantation, doctors use a whole cornea or parts of the five layers of the cornea to perform correction surgeries. In May 2013, Dr Harminder Dua discovered a sixth layer between the stroma and the descemet membrane which was named after him as the Dua's layer. In the PDEK technique, doctors take the innermost two layers of the cornea, along with the Dua's layer and graft it in the patient's eye.
In ophthalmology, glued intraocular lens or glued IOL is a surgical technique for implantation, with the use of biological glue, of a posterior chamber IOL in eyes with deficient or absent posterior capsules. A quick-acting surgical fibrin sealant derived from human blood plasma, with both hemostatic and adhesive properties, is used.
Corneal opacification is a term used when the human cornea loses its transparency. The term corneal opacity is used particularly for the loss of transparency of cornea due to scarring. Transparency of the cornea is dependent on the uniform diameter and the regular spacing and arrangement of the collagen fibrils within the stroma. Alterations in the spacing of collagen fibrils in a variety of conditions including corneal edema, scars, and macular corneal dystrophy is clinically manifested as corneal opacity. The term corneal blindness is commonly used to describe blindness due to corneal opacity.
Herbert Edward Kaufman is an American ophthalmologist who discovered idoxuridine, the first clinically useful antiviral agent; co-developed with William Bourne the clinical specular microscope to view the live corneal endothelium, co-developed timolol with Thomas Zimmerman, a new class of medications to treat glaucoma; corneal storage media for eye banks; natamycin, the first commercially available medication to treat fungal infections of the eye; co-developed with Tony Gasset the use of bandage contact lenses; and was involved in the first laser vision photorefractive keratectomy of the eye with Marguarite McDonald.